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Review

Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies

by
Sarah Julien
1,
Inès Challier
2,
Marine Malleter
1,
Fabienne Jouen
1,3,
Laurent Drouot
1 and
Olivier Boyer
1,3,*
1
INSERM U1234, PAn’THER FOCIS Center of Excellence, Université de Rouen, F-76000 Rouen, France
2
Department of Pharmacy, CHU Rouen, F-76000 Rouen, France
3
Department of Immunology and Biotherapy, CHU Rouen, F-76000 Rouen, France
*
Author to whom correspondence should be addressed.
Antibodies 2024, 13(1), 12; https://doi.org/10.3390/antib13010012
Submission received: 23 December 2023 / Revised: 10 January 2024 / Accepted: 2 February 2024 / Published: 7 February 2024

Abstract

Immune-mediated necrotizing myopathy (IMNM) is a rare and severe disease that corresponds to a specific entity of idiopathic inflammatory myopathy. Patients with IMNM suffer from proximal muscle weakness, and present high levels of creatine kinase and necrotic myofibers. Anti-Signal Recognition Particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies (HMGCR) have recently been identified in two thirds of patients with IMNM and are used as a hallmark of the disease. In this review, we provide a detailed description of these antibodies and the tests used to detect them in the serum of patients. Based on in vitro studies and mouse models of IMNM, we discuss the role of autoantibodies in the pathogenesis of the disease. Finally, in the light of the latest knowledge, we conclude with a review of recent therapeutic approaches in IMNM.
Keywords: immune-mediated necrotizing myopathy; myositis; anti-SRP; anti-HMGCR; autoantibodies immune-mediated necrotizing myopathy; myositis; anti-SRP; anti-HMGCR; autoantibodies

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MDPI and ACS Style

Julien, S.; Challier, I.; Malleter, M.; Jouen, F.; Drouot, L.; Boyer, O. Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies. Antibodies 2024, 13, 12. https://doi.org/10.3390/antib13010012

AMA Style

Julien S, Challier I, Malleter M, Jouen F, Drouot L, Boyer O. Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies. Antibodies. 2024; 13(1):12. https://doi.org/10.3390/antib13010012

Chicago/Turabian Style

Julien, Sarah, Inès Challier, Marine Malleter, Fabienne Jouen, Laurent Drouot, and Olivier Boyer. 2024. "Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies" Antibodies 13, no. 1: 12. https://doi.org/10.3390/antib13010012

APA Style

Julien, S., Challier, I., Malleter, M., Jouen, F., Drouot, L., & Boyer, O. (2024). Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies. Antibodies, 13(1), 12. https://doi.org/10.3390/antib13010012

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